Cardiac amyloidosis (CA) is a disease characterized by the deposition of misfolded protein deposits in the myocardial interstitium. Although advanced CA confers significant morbidity and mortality, the magnitude of deposition and ensuing clinical manifestations vary greatly. Thus, an improved understanding of disease pathogenesis at both cellular and functional levels would afford critical insights that may improve outcomes. This review will summarize contemporary therapies for the 2 major types of CA, transthyretin and light chain amyloidosis, and outline the capacity of imaging modalities to both diagnose CA, inform prognosis, and follow response to available therapies. We explore the current landscape of echocardiography, cardiac magnetic resonance, and bone scintigraphy in the assessment of functional and cellular parameters of dysfunction in CA throughout disease pathogenesis. Finally, we examine the impact of concurrent advances in both therapeutics and imaging on future research questions that improve our understanding of underlying disease mechanisms. Multimodal imaging in CA affords an indispensable tool to offer individualized treatment plans and improve outcomes in patients with CA.

中文翻译：

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心脏淀粉样变性：疾病活动和治疗反应的多模式成像

心脏淀粉样变性（CA）是一种以错误折叠的蛋白质沉积物在心肌间质中沉积为特征的疾病。尽管晚期 CA 具有显着的发病率和死亡率，但沉积的程度和随后的临床表现差异很大。因此，在细胞和功能水平上对疾病发病机制的更好理解将提供可能改善结果的关键见解。本综述将总结两种主要类型 CA（转甲状腺素蛋白和轻链淀粉样变性）的当代治疗方法，并概述成像方式在诊断 CA、告知预后和跟踪对可用疗法的反应方面的能力。我们探索超声心动图、心脏磁共振、和骨闪烁显像在整个疾病发病过程中评估 CA 功能障碍的功能和细胞参数。最后，我们研究了治疗学和影像学同时取得的进展对未来研究问题的影响，这些问题可以提高我们对潜在疾病机制的理解。CA 中的多模态成像为提供个性化治疗计划和改善 CA 患者的预后提供了不可或缺的工具。